Primary hyperparathyroidism (or PHPT) is a rare pediatric disease occurring in 1 case per 200 to 300 thousand children. The most common cause of PHPT in the adult population is a solitary parathyroid (PT) adenoma with up to 16% of PT adenomas having an ectopic location. There are no epidemiological data on pancreatic adenomas in pediatric population including atypically located ones, and clinical data are extremely scarce. This article describes a clinical observation of an adolescent patient with PHPT whose disease has been manifested with increased fragility and deformation of the bones of the skeleton. Only one year after the onset of primary symptoms a complex set of diagnostic measures was initiated, as a result of which a diagnosis was established: PHPT against the background of adenoma of the pancreas, ectopic in the left lobe of the thymus. The surgical treatment was carried out in the amount of thoracoscopic hemithymectomy which led to the relief of hyperparathyroidism. Despite the surgical intervention the systemic osteoporosis and persistent pathological changes in the bone skeleton that have been formed by the time of diagnosis require long-term observation and treatment of the patient by a pediatric endocrinologist, pediatric orthopedist and rehabilitologist. Thus, based on the presented clinical observation, the conclusion is as follows: in order to minimize the risk of disability in children and adolescents with PHPT it is extremely important to establish the correct diagnosis as early as possible coupled with the timely etiotropic and pathogenetic treatment.